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Original research article
Arrhythmias in adult patients with congenital heart disease and pulmonary arterial hypertension
  1. Maria Drakopoulou1,2,
  2. Heba Nashat1,
  3. Aleksander Kempny1,
  4. Rafael Alonso-Gonzalez1,
  5. Lorna Swan1,
  6. Stephen J Wort1,
  7. Laura C Price1,
  8. Colm McCabe1,
  9. Tom Wong1,
  10. Michael A Gatzoulis1,
  11. Sabine Ernst1,
  12. Konstantinos Dimopoulos1
  1. 1 Adult Congenital Heart Centre and National Centre for Pulmonary Hypertension, Royal Brompton Hospital, National Heart and Lung Institute, Imperial College London, London, UK
  2. 2 First Department of Cardiology, Hippokration Hospital, National and Kapodistrian Athens University, Athens, Greece
  1. Correspondence to Dr Konstantinos Dimopoulos, Adult Congenital Heart Centre, Royal Brompton and Harefield NHS Foundation Trust, SW3 6NP London, UK; k.dimopoulos02{at}


Objectives Approximately 5%–10% of adults with congenital heart disease (CHD) develop pulmonary arterial hypertension (PAH), which affects life expectancy and quality of life. Arrhythmias are common among these patients, but their incidence and impact on outcome remains uncertain.

Methods All adult patients with PAH associated with CHD (PAH-CHD) seen in a tertiary centre between 2007 and 2015 were followed for new-onset atrial or ventricular arrhythmia. Clinical variables associated with arrhythmia and their relation to mortality were assessed using Cox analysis.

Results A total of 310 patients (mean age 34.9±12.3 years, 36.8% male) were enrolled. The majority had Eisenmenger syndrome (58.4%), 15.2% had a prior defect repair and a third had Down syndrome. At baseline, 14.2% had a prior history of arrhythmia, mostly supraventricular arrhythmia (86.4%). During a median follow-up of 6.1 years, 64 patients developed at least one new arrhythmic episode (incidence 3.47% per year), mostly supraventricular tachycardia or atrial fibrillation (78.1% of patients). Arrhythmia was associated with symptoms in 75.0% of cases. The type of PAH-CHD, markers of disease severity and prior arrhythmia were associated with arrhythmia during follow-up. Arrhythmia was a strong predictor of death, even after adjusting for demographic and clinical variables (HR 3.41, 95% CI 2.10 to 5.53, p<0.0001).

Conclusions Arrhythmia is common in PAH-CHD and is associated with an adverse long-term outcome, even when managed in a specialist centre.

  • congenital heart disease

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  • SE and KD contributed equally.

  • Contributors All Authors have contributed to this manuscript.

  • Funding MD has received research grant from Elikar Foundation. AK, KD, RA-G and MAG have received unrestricted educational or research grants from Bayer, Pfizer, Actelion and GSK. Furthermore, they have received support from the Pulmonary Hypertension Association (UK) and the British Heart Foundation. KD, RA-G and MAG have acted as consultants to Pfizer, Actelion and GSK. SE has acted as a consultant to Biosense Webster and Stereotaxis. HN has received an educational grant from Actelion. LCP has educational grants from Actelion and GSK. SJW has received honoraria and travel grants from Bayer, Actelion and GSK. He has received research grants from Bayer and Actelion.

  • Competing interests None declared.

  • Patient consent Not required.

  • Provenance and peer review Not commissioned; externally peer reviewed.